Ectopia Lentis Syndrome:Ectopia lentis, with or without Systemic score ≥7 & with FBN1 not known, with Ao (Z ≥2 or Dissection) or no FBN1 = Ectopic Lentis Syndrome. (My interpretation: Ectopia lentis with aortic changes (Z ≥2 or Dissection) is Ectopia lentis syndrome. Systemic Score ≥7 or FBN1 positive are not essential, but may be present).

(* Caveat: Without discriminating features of Shprintzen-Goldberg Syndrome, Loeys-Dietz syndrome, or Vascular form of Ehler-Danlos Syndrome & after TGFBR1/2, Collagen biochemistry, COL3A1 testing if indicated. Other conditions/genes will emerge with time)